Paglietti Maria Giovanna, Esposito Irene, Goia Manuela, Rizza Elvira, Cutrera Renato, Bignamini Elisabetta
Pediatric Pulmonology & Respiratory Intermediate Care Unit, Sleep and Long-Term Ventilation Unit, Academic Department of Pediatrics, Bambino Gesù Children's Hospital, Rome, Italy.
Pediatric Pulmonology & Regional Reference Centre for Pediatric Respiratory Failure and Cystic Fibrosis, Regina Margherita's Hospital, AOU Città della Salute e della Scienza, Turin, Italy.
Front Pediatr. 2020 Jun 19;8:288. doi: 10.3389/fped.2020.00288. eCollection 2020.
Central hypoventilation (CH) is a quite rare disorder caused by some congenital or acquired conditions. It is featured by increased arterial concentration of serum carbon dioxide related to an impairment of respiratory drive. Patients affected by CH need to be treated by mechanical ventilation in order to achieve appropriate ventilation and oxygenation both in sleep and wakefulness. In fact, in severe form of Congenital Central Hypoventilation Syndrome (CCHS) hypercarbia can be present even during the day. Positive pressure ventilation via tracheostomy is the first therapeutic option in this clinical condition, especially in congenital forms. Non-Invasive ventilation is a an option that must be reserved for more stable clinical situations and that requires careful monitoring over time.
中枢性通气不足（CH）是一种由某些先天性或后天性疾病引起的极为罕见的病症。其特征是与呼吸驱动力受损相关的血清二氧化碳动脉浓度升高。患有CH的患者需要接受机械通气治疗，以便在睡眠和清醒状态下都能实现适当的通气和氧合。事实上，在严重形式的先天性中枢性通气不足综合征（CCHS）中，即使在白天也可能出现高碳酸血症。在这种临床情况下，经气管切开术进行正压通气是首要的治疗选择，尤其是对于先天性形式。无创通气是一种必须保留用于更稳定临床情况且需要长期仔细监测的选择。
